for orthopaedics resident

1. PAGET’S DISEASE OF
THE BONE
Seminar on 24/4/2018
Presented by dr. ravi diwakar rso

2. INTRODUCTION
The condition was initially described by Dr.
James Paget in 1877
 Also called as Osteitis Deformans
 Partial or complete involvement of a single
or multiple bones by exaggerated rates of
resorptive and osteogenic activity leading to
bony thickening and deformity.
. Schmorl believed that approximately 3% of
everyone above 40 years had osteitis
deformans

3. It has a predilection for the axial skeleton
 Pelvis>tibia > Femur > Skull>spine >clavicle
 But any bone may be affected
 Paget disease is common in Europe, North
America
 It is rare in Asia and Africa

4. ETIOLOGY
• UNKNOWN
• Occasionally hereditary influence is noted on
chromosome 18q
• On electron microscopy of bone biopsies has
demonstrated nuclear inclusions, similar to
those found in viral diseases (Paramyxoviridae
family) are found in the highly nucleated
osteoclasts.
• Endocrine and metabolic disturbances are
unlikely because despite extensive involvement ,
many bones are free of disease.

5. PATHOPHYSIOLOGY
• 3 Phases:
• i) Lytic
• Ii) mixed Lytic and Blastic
• iii) Sclerotic
• Different skeletal lesions may progress at
different rates.
• At a given time, multiple stages of disease may
be demonstrated in different skeletal regions of
same patient.

6. LYTIC PHASE
• Disease begins with lytic phase
• The bone is resorbed by osteoclasts that are
more numerous, larger, and have more nuclei
(upto 100)
• Bone turnover rate increased as much as
20times normal

7. Mixed Lytic and Blastic phase
• Rapid increase in bone formation from numerous
osteoblasts
• Morphologically osteoblasts are normal
• The newly formed bone is abnormal with collagen fibers
deposited in haphazard fashion rather than linear
• As osteoclastic and osteoblastic activity repeats, high
degree of bone turn over occurs.

8. Sclerotic Phase
• The bone formation dominates and has a disorganized woven
pattern and is weaker than normal bone.
• Woven pattern allows the bone marrow to be infiltrated by
excessive fibrous connective tissue and blood vessels leading
to hyper vascular bone state.
• Eventually osteoblastic activity also declines and enters a
sclerotic or burned-out phase.
• Continued bone resorption is minimal or absent.

9. Histology

10. Complications
• Fractures and bony deformity
• Secondary osteoarthritis ( when pagets disease around a joint)
• Neurological complications – nerve root compression and cauda
equina syndrome
• Skull involvement-
 deafnes
 vertigo
 tinnitus
 dental malocclusion
 basilar invagination
 Cranial nerve disorders
• Sarcomatous degeneration - Osteosarcoma
• Increased bone vascularity – high output cardiac failure

11. SURGICAL COMPLICATIONS:
• Highly vascular marrow – Profuse bleeding
• Structurally weak bone
• Spinal / Epidural Anesthesia may be difficult

12. Investigations
• Serum Alkaline phosphatase ( reflect osteogenic potetional of
osteoblast cell and extent of disease)
• Serum Acid phosphatase
• Urinary Markers – hydroxyproline,
deoxypyridinoline, C-telopeptide, Ntelopeptide
• Serum calcium and phosphate levels will be
normal

13. X-RAYS
• Long bones:
bowing
thickening of cortex, narrowing of medulla
honey combed or spongy, large dense bone
looser’s zone of transformation

14. Brim Sign
• thickening of the right pelvic brim (ileopectineal line) as
compared to the left

15. Skull
• osteitis circumscripta or cotton wool exudates

16. tam o' shanter sign
• widening of the diploic space and an overall
enlargement of the cranium

17. Pagets Spine
Picture frame sign Ivory Vertebra

18. Blade of Grass or Candle
flame sign
• begins as a subchondral area of lucency with
advancing tip of V-shaped osteolysis, extending
towards the diaphysis

19. Looser’s Zone

20. Scintigraphy
• Technetium 99 scan
shows increased
uptake in right
hemipelvis and spine

21. DIFFERENTIAL DIAGNOSIS:
• Osteomalacia
• Fibrous Dysplasia
• Multiple Myeloma
• osteopetrosis

22. TREATMENT
• Inactive lesions doesn’t require any intervention
• Goals of treatment:
Suppression of Active disease
Relief of Pain
Prevention of Deformity and fractures
High output cardiac dysfunction
Reducing the Sarcomatous transformation

23. Suppressive Agents
• BISPHOSPHONATES
• 2nd generation bisphosphonates like Tiludronate, Alendronate,
risendronate produces longer remission at lower doses.
 Pamidronate – 30mg IV/day over 3hours for 3days
 Zolidronic Acid- 5mg IV over 5 mins
• First choice where rapid mineralization is required
• in neurological symptoms, severe bone pain, risk of fracture
• prior to elective surgery
• Vitamin D and calcium supplements
• It normalizes the ALP in 6 months
• Bisphosphonates should not be used in patients with renal
impairment

24. Calcitonin
• Dosage – 100 IU / day SC/IM for 6-18 months
• reduced to 50 IU / day x 3/week
• Calcitonin therapy can temporarily arrest active
disease
 ALP, urine Hydroxyproline is reduced
 Positive Calcium balance
 High output heart failure is improved
 Bone pain relieved

25. • Surgical treatment is reserved for
fractures,
correction of bone deformity,
THR,
Spinal surgery
Preoperatively and postoperatively calcitonin
therapy gives good results and reduces
bleeding.

26. THANK YOU